The esthesioneuroblastoma (ENB) is a rare malignant sinonasal tumor of neuroectodermal origin. This study aimed to improve the understanding of the clinical features by reviewing the literature and analyzing the medical records of patients diagnosed with ENB in our institution between 2012 and 2019. A total of 6 cases of ENB were available for analysis. The mean age at the time of diagnosis was 36 years. The main complaints at presentation were the rhinologic signs. Tumors were classified as stage C of Kadisk in 3 cases and stage D in the others. According to TNM (modified by Dulguerov), 2 patients were T3N0M0, one T4N0M0, one T3N1M0, and two T4N1M0. The diagnosis of ENB was based on pathological examination. According to Hyams histological grading, low-grade lesions (Hyams I and II) were seen in 2 cases, high-grade undifferentiated lesions (Hyams III and IV) were seen in 4. Of the 6 patients, 4 received surgery. The surgical approaches mainly included an endoscopic endonasal resection in 2 cases, a cranial-facial resection surgery in 1 case, and an expanded endoscopic endonasal approach in combination with craniotomy in 1 case. Four patients received adjuvant radiotherapy (RT). RT dose ranged from 60 to 70 Gy. A total of 3 patients had lymph node metastasis and received RT of the neck. Chemotherapy was delivered in 2 patients. After a mean follow-up of 4.5 years, 4 patients were free of recurrence. Unfortunately, 1 patient died from a progressive disease 6 months after RT. ENB is a rare locally aggressive tumor of the nasosinusal cavities. The first-line treatment for resectable tumors should include primary surgical resection with adjuvant RT. However, this tumor remains of poor prognosis. Therefore, long-term close follow-up based on symptoms, endoscopy, and imaging is essential.
Les tumeurs neuroendocrines sont une entite rare des tumeurs du tractus genital feminin, elles representent 1,4% de tous les cancers invasifs du col de luterus. Ce sont des tumeurs rares, de mauvais pronostic. Les options therapeutiques sont souvent extrapolees a partir du cancer du poumon a petites cellules et detudes retrospectives limitees. Le traitement prefere est une approche multimodale associant chirurgie, radiotherapie et traitement systemique. Nous rapportons le cas dune patiente traitee pour une tumeur neuroendocrine du col uterin au service de radiotherapie du Centre Hospitalier Universitaire Hassan II de FES.
Paratesticular Rhabdomyosarcoma is a rare mesenchymal tumor. The Alveolar variant is the one with the worse prognosis. It comprehend 20% of cases. The treatment is multimodal combining surgery, chemotherapy and radiotherapy. Depending on the extent of disease and the staging group, the approach of treatment is different. Here we report the case of an alveolar Paratesticular rhabdomyosarcoma and review the literature regarding this unusual clinical entity.
Jugulo-tympanic paragangliomas are rare benign tumors arising from the neuroectodermal cells of the tympanic cavity and the dome of the jugular vein. Surgical resection has long been the first-line treatment. However, when this is not possible, radiotherapy remains a therapeutic alternative allowing local control with less morbidity. We report the case of a patient with an unresectable tympano-jugular paraganglioma, the symptomalagy was dominted by chronic headaches and right sided hypoacousie, and the treatment consisted of external radiation therapy with a total dose of 50 Gy in 25 fractions, one fraction per day, 2 Gy per fraction using the IMRT technique. The evolution was good with disappearance of clinical symptomatology, significant local control with less toxicity.
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